Family History Of Premature Death Research Articles

36 Five years on – failure to apply 2010 nice syncope guidelines in a london tertiary centre

Introduction NICE have recommended clear pathways for assessment of patients with transient loss of consciousness (TLoC), with the aim of reducing unnecessary investigations, the length of hospital admission and aiming for early specialist assessment and better patient outcomes. Methods A retrospective analysis of electronic case records for all patients referred by A and E for medical assessment in King’s College Hospital in July and August 2015 was performed in August 2016. To capture all potential patients, any referral including the words fall, collapse, seizure, syncope, LoC, cardiac arrest or blackout were screened. Patients who did not have TLoC were excluded based on prespecified criteria. The remaining patient records were evaluated against the 2010 NICE guideline audit tool. Results 2101 patients were referred over 2 months. 192 (9%) were possible TLoC on the basis of the initial referral. After medical assessment, 147 patients were found not to have TLoC. The most common reasons were absence of LoC (75 pts), epileptic seizure with known epilepsy (24 pts) and drug or alcohol intoxication (17 pts). After screening, there were 45 patients (2%,) with potential TLoC. No patient with TLoC had the all the NICE-mandated minimum data recorded. Unrecorded data included high risk features that should be the basis for TLoC admissions; family history of premature death (40%), collapse during exertion (20%), new or unexplained dyspnoea (18%). Although 82% had a CXR and 76% received CT head, 84% had no recorded postural BP and 24% had no record of an ECG. Only 16% were referred for inpatient evaluation by a cardiologist. The mean length of stay was 7 days, equivalent to 5 bed-years on an annualised basis. However, 20% were discharged without a diagnosis, and 36% did not have any outpatient follow up. Conclusions Despite clear guidelines, syncope patients still do not receive appropriate initial evaluation, investigation or diagnosis, even in a large tertiary centre with ready access to specialist assessment. Hospital length of stay is long, yet simple diagnostic tests (such as ECGs and postural BPs) are commonly not performed. Screening of all medical referrals based on simple criteria results in a relatively small number of patients with TLoC. Early specialist involvement in patients with syncope would be practically feasible, and would potentially result in better patient outcomes.

Risk of Cardiomyopathy in Younger Persons With a Family History of Death from Cardiomyopathy: A Nationwide Family Study in a Cohort of 3.9 Million Persons.

Recommendations for presymptomatic screening of relatives of cardiomyopathy patients are based on findings from tertiary centers. Cardiomyopathy inheritance patterns are fairly well understood, but how cardiomyopathy in younger persons (<50 years) aggregates in families at the population level is unclear. In a nationwide cohort, we examined the risk of cardiomyopathy by family history of premature death (<60 years) from cardiomyopathy. By linking Danish national register data, we constructed a cohort of 3.9 million persons born from 1950 to 2008. We ascertained family history of premature (<60 years) death from cardiomyopathy or other conditions, and cohort members were followed from 1977 to 2008 for cardiomyopathy diagnosed at <50 years. We identified 3890 cardiomyopathies in 89 million person-years of follow-up. Using Poisson regression, we estimated incidence rate ratios for cardiomyopathy by family history of premature death. Premature cardiomyopathy deaths in first- and second-degree relatives were associated with 29- and 6-fold increases in the rate of cardiomyopathy, respectively. If the first-degree relative died aged <35 years, the rate of cardiomyopathy increased 100-fold; given ≥2 premature deaths in first-degree relatives, the rate increased more than 400-fold. In contrast, a family history of premature death from other cardiac or noncardiac conditions increased the rate of cardiomyopathy 3-fold at most. A family history of premature cardiomyopathy death was associated with an increase in risk of cardiomyopathy ranging from 6- to 400-fold, depending on age, kinship, gender and number of affected family members. Our general population-based results support recommendations for presymptomatic screening of relatives of cardiomyopathy patients.

Opioid addiction agonist therapy and the QT prolongation phenomenon: state of the science and evolving research questions

Opioid addiction agonist therapy and the <scp>QT</scp> prolongation phenomenon: state of the science and evolving research questions

Family History of Premature Death and Risk of Early Onset Cardiovascular Disease

The purpose of this study was to examine the effect of a family history of premature death, cardiovascular death in particular, on the risk of early cardiovascular disease. Studies suggest that fatal cardiovascular events and less severe cardiovascular diseases may co-occur in families. Consequently, a family history of premature death may indicate a familial cardiac frailty that predisposes to early cardiovascular disease. We ascertained family history of premature death (age <60 years) in all individuals born in Denmark from 1950 to 2008 and followed this cohort for early cardiovascular disease (age <50 years). Using Poisson regression, we estimated incidence rate ratios (IRRs) reflecting the effect of premature death in the family on early cardiovascular disease risk. Among 3,985,301 persons followed up for 89,294,258 person-years, 129,825, 31,172, and 5,214 were diagnosed with any early cardiovascular disease, ischemic heart disease, and ventricular arrhythmia, respectively. IRRs for these conditions given a history of premature cardiovascular death in first-degree relatives were 1.72 (95% confidence interval [CI]: 1.68 to 1.77), 2.21 (95% CI: 2.11 to 2.31), and 1.94 (95% CI: 1.70 to 2.20), respectively. With ≥2 cardiovascular deaths in a family, corresponding IRRs were 3.30 (95% CI: 2.77 to 3.94), 5.00 (95% CI: 3.87 to 6.45), and 6.18 (95% CI: 3.32 to 11.50). The IRR for any early cardiovascular disease given a family history of premature noncardiovascular death was significantly lower, 1.12 (95% CI: 1.10 to 1.14) (p(cardiac vs. noncardiac) < 0.0001). Family history of premature cardiovascular death was consistently and significantly associated with a risk of early cardiovascular disease, suggesting an inherited cardiac vulnerability. These results should be kept in mind when assessing cardiovascular disease risk in persons with a family history of premature cardiovascular death.

Guidelines for the Diagnosis and Management of Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an uncommon inherited myocardial disorder characterised by fibro-fatty inflammation affecting the right and left ventricles. It most commonly presents with palpitations or syncope but sudden death may occur, especially in young males. Diagnosis is not possible with a single test and may be difficult. Task Force criteria agreed in 1994 comprise major and minor criteria spanning structural abnormalities, ECG appearances, arrhythmias, family history of premature death and myocardial histology. Modified criteria were introduced in 2010 to improve sensitivity. Arrhythmogenic right ventricular cardiomyopathy is a desmosomal disease. Mutations have been detected in five desmosomal genes, most frequently in plakophilin-2 (PKP2) and multiple mutations are also reported. Antiarrhythmic drugs such as sotalol and amiodarone may improve symptoms but are unproven to increase survival. An implantable defibrillator is appropriate in individuals surviving cardiac arrest or sustained ventricular tachycardia, but there is not yet consensus about prophylactic treatment of Task Force positive but asymptomatic individuals. Arrhythmogenic right ventricular cardiomyopathy is more common than previously believed. Preliminary evidence supports improved sensitivity without loss of specificity using the revised Task Force criteria. The genetics of the disease are complex but should ultimately advance diagnosis and management.

Process of Care and Prescribing Practices for Hypertension in Public and Private Primary Care Clinics in Malaysia

This study aimed to compare the process of care and the choice of antihypertensive medications used in both public and private primary care clinics in Malaysia. A cross-sectional survey was completed in 2008 on randomly selected 100 public health clinics and 114 private primary care clinics in Malaysia. A total of 4076 patient records, 3753 (92.1%) from public clinics and 323 (7.9%) from private clinics were analyzed. Less than 80% of the records documented the recommended clinical and laboratory assessments. The rates of documentation for smoking status, family history of premature death, retinal assessment, and urine albumin tests were lower in public clinics. Overall, 21% of the prescription practices were less than optimal. The process of care and the use of antihypertensive medications were not satisfactory in both settings.

The Clustering of Premature Deaths in Families

Infant deaths cluster in families, but beyond infancy, little is known about familial aggregation of premature deaths. We hypothesized that an individual's risk of premature death would be influenced by prior premature deaths of any age in family members. For all Danish residents registered in the Civil Registration System, 1968-2005, information was linked to the Causes of Death Register and the Danish Family Relation Database, yielding a cohort of 4,870,821 persons linked to one or more relatives (93 million persons-years and 73,278 deaths). We used log-linear Poisson regression to estimate mortality rate ratios (relative risks) for premature death (before age 40 years) in persons with a family history of premature death, compared with persons without such a history. Persons with a family history of premature death were 46% more likely themselves to die prematurely than persons without such a family history (relative risk [RR] = 1.46 [95% confidence interval = 1.42-1.50]). Relative risks were higher for concordant age at death, a close kinship relation, and similar causes of death. As expected, certain natural causes clustered among first-degree relatives (RRs ranged from 1.81-618), but unnatural causes of death, such as nonsimultaneous motor vehicle injuries, other injuries, and suicides, also clustered (RRs = 1.80, 3.53, and 4.23, respectively). Previous family history of premature death in a first-degree relative also increased the risk of dying from another cause (overall RR = 2.08 in infancy and 1.33 between ages 1 and 39). Premature deaths cluster in families, for both similar and dissimilar causes.

Prevalence of arrhythmias during 24-hour electrocardiographic monitoring and exercise testing in patients with obstructive and nonobstructive hypertrophic cardiomyopathy.

Many patients with hypertrophic cardiomyopathy die suddenly and unexpectedly, a significant number perhaps due to arrhythmia. Of 100 patients initially evaluated for signs or symptoms suggestive of heart disease or a family history of hypertrophic cardiomyopathy, 51 were selected solely because they met the echocardiographic criteria for the disease, and 49 patients were selected primarily because they had: 1) normal sinus rhythm despite left atrial enlargement, 2) a history of syncope, 3) a family history of premature death, or 4) a history of paroxysmal atrial fibrillation. All 100 patients were studied by 24-hour ambulatory electrocardiographic monitoring and 74 of them also underwent treadmill exercise testing. More than 50% of patients in repetitive ventricular premature depolarizations, including 19% who had ventricular tachycardia. Monitoring was superior to exercise testing for exposing these arrhythmias. Two patients experienced cardiac arrest within 2 months of monitoring; in each, monitoring had revealed ventricular tachycardia. Two patients with paroxysms of supraventricular tachycardia during monitoring developed fixed atrial fibrillation within 1 year. These preliminary observations suggest that monitoring may help identify patients at increased risk for significant arrhythmic events.